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background_position=&#8221;top_left&#8221; background_repeat=&#8221;repeat&#8221; global_colors_info=&#8221;{}&#8221;]<\/p>\n<h3>\u9700\u8981\u76e3\u6e2c<\/h3>\n<p>\u4f5c\u70ba\u4e00\u7a2e\u591a\u7cfb\u7d71\u75be\u75c5\uff0c\u7121\u8ad6\u60a3\u8005\u53ef\u80fd\u4f7f\u7528\u4f55\u7a2e\u6cbb\u7642\u65b9\u6cd5\uff0c\u90fd\u5fc5\u9808\u5bc6\u5207\u76e3\u6e2c Erdheim-Chester \u75c5\u3002\u5efa\u8b70\u4efb\u4f55\u88ab\u8a3a\u65b7\u70ba ECD \u7684\u4eba\u5b9a\u671f\u9032\u884c\u4ee5\u4e0b\u6aa2\u67e5\uff0c\u4ee5\u76e3\u6e2c\u75be\u75c5\u548c\u6f5b\u5728\u7684\u6cbb\u7642\u526f\u4f5c\u7528\uff1a <\/p>\n<ul>\n<li><strong>\u5fc3\u81df\u76e3\u6e2c\u3002<\/strong>  \u5c0d\u65bc\u5fc3\u81df\u53d7\u7d2f\u7684\u60a3\u8005\uff0c\u61c9\u6bcf 3 \u500b\u6708\u9032\u884c\u4e00\u6b21\u984d\u5916\u7684\u5fc3\u81df\u548c\/\u6216\u4e3b\u52d5\u8108\u8a55\u4f30\uff0c\u76f4\u5230\u6cbb\u7642\u540e\u75c5\u60c5\u7a69\u5b9a\u3002\u6b64\u6642\uff0c\u7814\u7a76\u901a\u5e38\u5ef6\u9577\u81f3\u6bcf\u516d\u500b\u6708\u4e00\u6b21\u3002\u63a5\u53d7 BRAF \u6291\u88fd\u5291\u6cbb\u7642\u7684\u60a3\u8005\u9084\u9700\u8981\u5b9a\u671f\u9032\u884c\u5fc3\u96fb\u5716\u6aa2\u67e5\u3002  <\/li>\n<li><strong>CNS \u76e3\u6e2c\u3002<\/strong>  \u5982\u679c\u767c\u73fe\u6216\u61f7\u7591 CNS \u53d7\u7d2f\uff0c\u61c9\u6bcf 3 \u500b\u6708\u91cd\u8907\u4e00\u6b21\u8166\u90e8 MRI \u8a55\u4f30\u548c\u5c0d\u5c0f\u8166\u7684\u7279\u7570\u6027\u7814\u7a76\uff0c\u76f4\u5230\u6cbb\u7642\u5f8c\u75c5\u60c5\u7a69\u5b9a\u3002\u6b64\u6642\uff0c\u7814\u7a76\u901a\u5e38\u6e1b\u5c11\u5230\u6bcf 6 \u5230 12 \u500b\u6708\u4e00\u6b21\u3002 <\/li>\n<li><strong>\u67d0\u4e9b\u60a3\u8005\u53ef\u80fd\u6703\u8003\u616e\u7684\u6383\u63cf\u3002<\/strong>  PET\/CT \u6383\u63cf\u76e3\u6e2c\u75be\u75c5\u7684\u6d3b\u52d5\u548c\u7bc4\u570d\u4ee5\u53ca\u5c0d\u6cbb\u7642\u7684\u53cd\u61c9\u3002\u6839\u64da\u91ab\u751f\u7684\u5efa\u8b70\uff0c\u53ef\u4ee5\u8003\u616e\u6bcf 3 \u5230 6 \u500b\u6708\u9032\u884c\u4e00\u6b21\u9019\u4e9b\u6e2c\u8a66\uff0c\u76f4\u5230\u75be\u75c5\u7a69\u5b9a\u4e0b\u4f86\u3002 <\/li>\n<li><strong>\u5782\u9ad4\/\u6fc0\u7d20\u76e3\u6e2c\u3002<\/strong>  \u5efa\u8b70\u5c0d\u4e00\u4e9b\u60a3\u8005\u9032\u884c\u8a55\u4f30\u5782\u9ad4\u529f\u80fd\u3002\u4e3b\u6cbb\u91ab\u5e2b\u5c07\u8003\u616e\u662f\u5426\u9700\u8981\u7531\u5167\u5206\u6ccc\u5b78\u5bb6\uff08\u6fc0\u7d20\u5c08\u5bb6\uff09\u9032\u884c\u6fc0\u7d20\u6c34\u5e73\u6aa2\u6e2c\uff0c\u5305\u62ec\u777e\u916e\u3001ADH\u3001\u7532\u72c0\u817a\u6fc0\u7d20\u3001\u80f0\u5cf6\u7d20\u3001ACTH \u548c PTH \u6aa2\u6e2c\u3002 <\/li>\n<li><strong>\u76ae\u819a\u76e3\u6e2c\u3002<\/strong>  \u5c0d\u65bc\u4f7f\u7528 BRAF \u548c \/\u6216 MEK \u6291\u88fd\u5291\u7684\u60a3\u8005\uff0c\u61c9\u5728\u958b\u59cb\u6cbb\u7642\u540e\u6bcf 3 \u500b\u6708\u9032\u884c\u4e00\u6b21\u76ae\u819a\u6aa2\u67e5\uff0c\u4e00\u65e6\u75be\u75c5\u7a69\u5b9a\uff0c\u61c9\u5ef6\u9577\u81f3\u6bcf 6 \u500b\u6708\u4e00\u6b21\u3002<\/li>\n<li><strong>\u773c\u775b\u76e3\u6e2c\u3002<\/strong>  \u5c0d\u65bc\u670d\u7528MEK\u6291\u88fd\u5291\u7684\u60a3\u8005\uff0c\u5efa\u8b70\u5728\u958b\u59cb\u6cbb\u7642\u540e4-6\u5468\u5167\u9032\u884c\u5c08\u9580\u7684\u8996\u7db2\u819c\u6563\u77b3\u6aa2\u67e5\uff0c\u7136\u5f8c\u53ef\u4ee5\u6bcf3-6\u500b\u6708\u6216\u6839\u64da\u81e8\u5e8a\u6307\u5fb5\u9032\u884c\u96a8\u8a2a\u3002<\/li>\n<\/ul>\n<h3>\u7269\u7406\uff08\u7269\u7406\uff09\u7642\u6cd5<\/h3>\n<p>\u6b64\u5916\uff0c ECD \u60a3\u8005\u9700\u8981\u5118\u65e9\u958b\u59cb\u7269\u7406\uff08\u7269\u7406\uff09\u6cbb\u7642\u670d\u52d9\u3002\u6839\u64da\u60a3\u8005\u7684\u7665\u72c0\uff0c\u9019\u53ef\u80fd\u5305\u62ec\u7269\u7406\u6cbb\u7642\u3001\u8077\u696d\u6cbb\u7642\u548c\/\u6216\u8a00\u8a9e\/\u541e\u54bd\u6cbb\u7642\u3002\u60a3\u8005\u548c\u91ab\u751f\u5831\u544a\u8aaa\uff0c\u9019\u662f\u6cbb\u7642\u7ba1\u7406\u7684\u91cd\u8981\u7d44\u6210\u90e8\u5206\uff0c\u4e0d\u61c9\u88ab\u5ffd\u8996\u3002  <\/p>\n<h3>\u5fc3\u7406\u8003\u616e<\/h3>\n<p>\u6700\u5f8c\uff0c\u4efb\u4f55\u9047\u5230\u5fc3\u7406\u6216\u793e\u6703\u5fc3\u7406\u554f\u984c\u7684\u60a3\u8005\u90fd\u61c9\u8a72\u8207\u4ed6\u5011\u7684\u91ab\u751f\u8a0e\u8ad6\u9019\u500b\u554f\u984c\u3002\u9019\u5c0d\u65bc ECD \u60a3\u8005\u4f86\u8aaa\u4e26\u4e0d\u5c11\u898b\uff0c\u6cbb\u7642\u53ef\u4ee5\u63d0\u4f9b\u8aac\u660e\u3002\u9f13\u52f5\u60a3\u8005\u7e7c\u7e8c\u9032\u884c\u4ed6\u5011\u559c\u6b61\u7684\u793e\u4ea4\u4e92\u52d5\u548c\u4f11\u9592\u6d3b\u52d5\u3002\u901a\u904e\u63d0\u4f9b\u7684\u8a08\u5283\u5c0b\u6c42\u8aee\u8a62\u548c\u53c3\u8207 ECDGA \u4e5f\u88ab\u767c\u73fe\u53ef\u4ee5\u8aac\u660e\u60a3\u8005\u3002   <\/p>\n<h3>\u751f\u5b58\u9700\u6c42<\/h3>\n<ul>\n<li><strong>\u75b2\u52de<\/strong> \u662f ECD \u60a3\u8005\u7684\u4e00\u500b\u91cd\u8981\u554f\u984c\uff0c\u901a\u5e38\u662f\u7531\u65bc\u75be\u75c5\u672c\u8eab\u548c\u6cbb\u7642\u526f\u4f5c\u7528\u9020\u6210\u7684\u3002\u5118\u7ba1\u5f88\u5c11\u6709\u5e72\u9810\u63aa\u65bd\u88ab\u9a57\u8b49\u53ef\u4ee5\u6539\u5584\u9019\u500b\u554f\u984c\uff0c\u4f46\u63a8\u85a6\u4e86\u5e7e\u7a2e\u65b9\u6cd5\uff1a\n<ul>\n<li><strong>\u935b\u7149<\/strong>\uff1a\u81e8\u5e8a\u91ab\u751f\u61c9\u5f37\u70c8\u5efa\u8b70\u5118\u53ef\u80fd\u9032\u884c\u935b\u7149\uff08\u6709\u6c27\u904b\u52d5\u3001\u963b\u529b\u904b\u52d5\u6216\u7d44\u5408\u904b\u52d5\uff09\u3002<\/li>\n<li><strong>\u8a8d\u77e5\u884c\u70ba\u7642\u6cd5 \uff08CBT\uff09\uff1a<\/strong>\u81e8\u5e8a\u91ab\u751f\u61c9\u63a8\u85a6 CBT\uff0c\u6709\u6216\u6c92\u6709\u50ac\u7720\u3002<\/li>\n<li><strong>\u57fa\u65bc\u6b63\u5ff5\u7684\u8a08\u5283<\/strong>\uff1a \u81e8\u5e8a\u91ab\u751f\u61c9\u63a8\u85a6\u57fa\u65bc\u6b63\u5ff5\u7684\u8a08\u5283\uff0c\u4ee5\u6e1b\u5c11\u75b2\u52de\u548c\u8207\u764c\u75c7\u76f8\u95dc\u7684\u58d3\u529b\u3002<\/li>\n<li><strong>\u592a\u6975\u62f3\u6216\u6c23\u529f<\/strong>\uff1a\u5e7e\u9805\u7814\u7a76\u8868\u660e\uff0c\u5728\u6e1b\u8f15\u764c\u75c7\u60a3\u8005\u7684\u75b2\u52de\u65b9\u9762\u53d6\u5f97\u4e86\u7a4d\u6975\u6210\u679c\u3002<\/li>\n<li><strong>\u85e5\u7269\u5e72\u9810<\/strong>\uff1a\u7cbe\u795e\u8208\u596e\u5291\u5c1a\u672a\u986f\u793a\u5c0d\u764c\u75c7\u60a3\u8005\u7684\u75b2\u52de\u6709\u76ca\u3002\u6297\u6291\u9b31\u6cbb\u7642\u53ef\u7528\u65bc\u6709\u6291\u9b31\u7665\u72c0\u7684\u60a3\u8005\uff0c\u5118\u7ba1\u5b83\u672c\u8eab\u4e0d\u6703\u6539\u5584\u75b2\u52de\u3002 <\/li>\n<\/ul>\n<\/li>\n<li><strong>\u795e\u7d93\u6b98\u75be\u3002<\/strong>  \u652f\u63f4\u6027\u8b77\u7406\u8a08\u5283\u5c0d\u65bc\u8207\u7d44\u7e54\u7d30\u80de\u589e\u591a\u75c7\u76f8\u95dc\u7684\u795e\u7d93\u529f\u80fd\u969c\u7919\u60a3\u8005\u81f3\u95dc\u91cd\u8981\u3002\u8077\u696d\u6cbb\u7642\u548c\u7269\u7406\u6cbb\u7642\u662f\u63d0\u9ad8\u751f\u6d3b\u8cea\u91cf\u7684\u5f37\u5236\u6027\u8981\u6c42\u3002\u795e\u7d93\u79d1\u91ab\u751f\u8207\u652f\u63f4\u6027\u8b77\u7406\u5718\u968a\u5408\u4f5c\uff0c\u5c0d\u65bc\u6839\u64da\u60a3\u8005\u7684\u6b98\u75be\u60c5\u6cc1\u63d0\u4f9b\u500b\u4eba\u5316\u8b77\u7406\u8a08\u5283\u81f3\u95dc\u91cd\u8981\u3002\u9019\u5169\u500b\u554f\u984c\u90fd\u5fc5\u9808\u8207 ECD \u5c08\u5bb6\u8a0e\u8ad6\uff0c\u4ee5\u5340\u5206\u8207\u764c\u75c7\u672c\u8eab\u76f8\u95dc\u7684\u75b2\u52de\u6216\u6b98\u75be\u4ee5\u53ca\u6cbb\u7642\u4e0d\u8010\u53d7\u6216\u526f\u4f5c\u7528\u3002[\u9019\u4e9b\u5efa\u8b70\u6e90\u81ea ASCO \u91dd\u5c0d\u764c\u75c7\u5016\u5b58\u8005\u7684\u5831\u544a\u3002    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